Transsphenoidal encephaloceles are rare and the transsellar variety is the least common. We present a 1-year-old male patient with transsellar transsphenoidal encephalocele which herniated into the oral cavity through the congenital split palate. The patient was operated on using a combined transcranial and transpalatal approach without mortality or permanent morbidity. Clinical findings, imaging reviews, surgical repair techniques and postoperative morbidity are discussed with the relevant literature. We conclude that repair of a transsphenoidal encephalocele should be coordinated between a team of neurosurgeons and otorhinolaryngologists. Our surgical outcome supports a transcranial approach for the treatment of these difficult lesions, with transpalatal dissection and exposure.